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Blood, 1 September 2002, Vol. 100, No. 5, pp. 1774-1779
IMMUNOBIOLOGY
Expression patterns of Th1 and Th2 cytokine genes in childhood
idiopathic thrombocytopenic purpura (ITP) at presentation and their
modulation by intravenous immunoglobulin G (IVIg) treatment: their role
in prognosis
Athanasia Mouzaki,
Maria Theodoropoulou,
Ioannis Gianakopoulos,
Vassiliki Vlaha,
Maria-Christina Kyrtsonis, and
Alice Maniatis
From the Laboratory Hematology and Transfusion Medicine
and Department of Pediatrics, School of Medicine, Patras University,
Patras, Greece, and Hematology Unit, Laikon Hospital, Athens, Greece.
Childhood idiopathic thrombocytopenic purpura (ITP) resolves
usually after the first episode, although it may recur, and in 10% to
20% of patients develops into a chronic disorder. Evidence of the
immunoregulatory role of Th1/Th2 responses in autoimmune diseases
prompted us to perform a prospective study of Th1/Th2 gene expression
profiles and transforming growth factor  (TGF-) plasma levels in
18 children (median age, 6.4 years) with acute ITP, before and after
intravenous immunoglobulin G (IVIg) infusion, and during a follow-up
period (0.5-5 years). Initially, 12 of 18 patients had either low
Th0/Th1 plus interleukin 10 (IL-10) or no in vivo cytokine gene
expression (0). At 24 hours after IVIg infusion this pattern became 0 or Th2 (9 of 12) or remained low Th0/Th1 (3 of 12). During follow-up
these patients did not relapse and maintained 0 or Th2 pattern without
IL-10. Of the remaining 6 patients, 4 presented with a Th1 or Th0/Th1
pattern plus IL-10 that persisted after IVIg treatment (although
interferon  [IFN-] expression diminished) and stabilized to Th1
plus IL-10 at follow-up, which was marked by infrequent episodes of
ITP. Two patients presenting with a strict Th1 pattern characterized by
high expression of IFN-, which remained unchanged after IVIg and at
follow-up, can be characterized as chronic ITP. TGF- plasma levels
were low in patients with active disease and increased in remission.
Overall, acute ITP presents with Th1, Th0/Th1, or 0 in vivo cytokine
gene expression. Stable remission is associated with a 0 or Th2
pattern. A 0 or Th2 pattern after IVIg gave the best prognosis, whereas
sustained high expression of IFN- and refractoriness to IVIg were
the main indicators of poor prognosis.
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